Acquired Angioedema due to C1-Inhibitor Deficiency: A Challenging Condition

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorVALLE, Solange Oliveira Rodrigues
dc.contributor.authorALONSO, Maria Luiza Oliva
dc.contributor.authorDORTAS JR., Sergio Duarte
dc.contributor.authorGOUDOURIS, Ekaterini Simoes
dc.contributor.authorCARVALHO, Ana Luiza Ribeiro Bard de
dc.contributor.authorCAPELO, Albertina Varandas
dc.contributor.authorMANSOUR, Eli
dc.contributor.authorBERNARDES, Ana Flavia
dc.contributor.authorLEITE, Luiz Fernando Bacarini
dc.contributor.authorGIAVINA-BIANCHI, Pedro
dc.contributor.authorAUN, Marcelo Vivolo
dc.contributor.authorFERRIANI, Mariana Paes Leme
dc.contributor.authorARRUDA, Luisa Karla
dc.contributor.authorGRUMACH, Anete Sevciovic
dc.date.accessioned2023-06-21T14:06:26Z
dc.date.available2023-06-21T14:06:26Z
dc.date.issued2022
dc.description.abstractBackground: Acquired deficiency of C1 inhibitor (AAE-C1-INH) is a very rare cause of recurrent angioedema, with few cases reported in the literature. We aimed to describe a series of patients with AAE-C1-INH who were diagnosed and received care at angioedema reference centers in Brazil, affiliated to the Brazilian Group of Studies on Hereditary Angioedema. Methods: Fourteen patients from 8 Brazilian Angioedema Reference Centers, diagnosed with AAE-C1-INH, were included in this study. Clinical data collected included sex, date of birth, date of onset of symptoms, date of diagnosis, plasma levels of antigenic and/or functional C1-INH, levels of C4 and C1q, location and treatment of angioedema attacks, long-term prophylaxis, associated diseases, and definitive treatment. Results: Fourteen patients were identified with AAE-C1-INH. Most patients (10/14; 71.4%) were female. The median age at onset of symptoms was 56.5 years (range, 14-74 years; interquartile range [IQR], 32-64 years), and median age at diagnosis was 58.0 years (range, 20-76 years; IQR, 38-65 years), with a median time until diagnosis of 2 years (range, 0-6 years; IQR, 1-3 years). The most common manifestations were cutaneous (face, eyelids, lips, trunk, hands, feet, and genitals). Most patient had low levels of C4 (13/14; 92.8%) and of antigenic C1-INH (8/14; 57.1%). Four had decreased functional activity of C1-INH (4/7; 57.1%) and C1q levels were low in 5 patients (5/12; 41.6%). Underlying diseases were identified in all 14 patients, with lymphoma of the splenic marginal zone and monoclonal gammopathy of undetermined significance being the most frequent. Nine patients (64.2%) needed long-term prophylactic treatment for recurrent angioedema and 5 patients (46.7%) required treatment for angioedema attacks. Most of them (12/14; 85.7%) had resolution of angioedema. Conclusion: Therapy of AAE-C1-INH aims to control symptoms; however, diagnosis and treatment of the underlying disease, when present, should be an important target and may lead to the resolution of angioedema in patients with AAE-C1-INH.eng
dc.description.indexMEDLINE
dc.description.indexPubMed
dc.description.indexWoS
dc.description.indexScopus
dc.identifier.citationINTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY, v.183, n.5, p.572-577, 2022
dc.identifier.doi10.1159/000521646
dc.identifier.eissn1423-0097
dc.identifier.issn1018-2438
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/53827
dc.language.isoeng
dc.publisherKARGEReng
dc.relation.ispartofInternational Archives of Allergy and Immunology
dc.rightsrestrictedAccesseng
dc.rights.holderCopyright KARGEReng
dc.subjectAcquired angioedemaeng
dc.subjectBradykinin-mediated angioedemaeng
dc.subjectC1-inhibitor autoantibodieseng
dc.subjectC1-inhibitor deficiencyeng
dc.subjectLymphoproliferative diseaseseng
dc.subject.otherhereditaryeng
dc.subject.otherdiagnosiseng
dc.subject.otherefficacyeng
dc.subject.wosAllergyeng
dc.subject.wosImmunologyeng
dc.titleAcquired Angioedema due to C1-Inhibitor Deficiency: A Challenging Conditioneng
dc.typearticleeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.author.externalVALLE, Solange Oliveira Rodrigues:Univ Fed Rio de Janeiro UFRJ, Hosp Univ Clementino Fraga Filho, Dept Internal Med, Immunol Serv, Rio De Janeiro, Brazil
hcfmusp.author.externalALONSO, Maria Luiza Oliva:Univ Fed Rio de Janeiro UFRJ, Hosp Univ Clementino Fraga Filho, Dept Internal Med, Immunol Serv, Rio De Janeiro, Brazil
hcfmusp.author.externalDORTAS JR., Sergio Duarte:Univ Fed Rio de Janeiro UFRJ, Hosp Univ Clementino Fraga Filho, Dept Internal Med, Immunol Serv, Rio De Janeiro, Brazil
hcfmusp.author.externalGOUDOURIS, Ekaterini Simoes:Univ Fed Rio de Janeiro UFRJ, Fac Med, Dept Pediat, Rio De Janeiro, Brazil
hcfmusp.author.externalCARVALHO, Ana Luiza Ribeiro Bard de:Univ Fed Rio de Janeiro UFRJ, Hosp Univ Clementino Fraga Filho, Dept Internal Med, Immunol Serv, Rio De Janeiro, Brazil
hcfmusp.author.externalCAPELO, Albertina Varandas:Univ Fed Estado RJ UNIRIO, Hosp Univ Gaffree & Guinle HUGG, Rio De Janeiro, Brazil
hcfmusp.author.externalMANSOUR, Eli:Univ Campinas UNICAMP, Sch Med Sci, Dept Internal Med, Allergy & Immunol, Campinas, Brazil
hcfmusp.author.externalBERNARDES, Ana Flavia:Univ Campinas UNICAMP, Sch Med Sci, Dept Internal Med, Allergy & Immunol, Campinas, Brazil
hcfmusp.author.externalLEITE, Luiz Fernando Bacarini:Irmandade Santa Casa Misericordia Sao Paulo, Immunodeficiency & Allergy Unit, Pediat Dept, Sao Paulo, Brazil
hcfmusp.author.externalFERRIANI, Mariana Paes Leme:Univ Sao Paulo, Ribeirao Preto Med Sch, Dept Med, Ribeirao Preto, Brazil
hcfmusp.author.externalARRUDA, Luisa Karla:Univ Sao Paulo, Ribeirao Preto Med Sch, Dept Med, Ribeirao Preto, Brazil
hcfmusp.author.externalGRUMACH, Anete Sevciovic:Ctr Univ FMABC, Fac Med, Clin Immunol, Santo Andre, SP, Brazil
hcfmusp.citation.scopus0
hcfmusp.contributor.author-fmusphcPEDRO FRANCISCO GIAVINA-BIANCHI JUNIOR
hcfmusp.contributor.author-fmusphcMARCELO VIVOLO AUN
hcfmusp.description.beginpage572
hcfmusp.description.endpage577
hcfmusp.description.issue5
hcfmusp.description.volume183
hcfmusp.origemWOS
hcfmusp.origem.pubmed35325890
hcfmusp.origem.scopus2-s2.0-85128511741
hcfmusp.origem.wosWOS:000989502500010
hcfmusp.publisher.cityBASELeng
hcfmusp.publisher.countrySWITZERLANDeng
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